Hormonal triggers for porphyria include:
(1) early pregnancy
(2) in the puerperium
(3) taking oral contraceptives
If a woman does have porphyria then she should receive genetic counseling about the risk to any offspring specific to the type of porphyria that she has inherited.
Low risk pregnancy:
(1) no history of acute attack
(2) normal porphyrin excretion
(3) free of symptoms for at least 18 months
High risk pregnancy:
(1) severe disease with frequent or severe acute attacks
(2) frequent vomiting with dehydration
Recommendations:
(1) Treat nausea and vomiting aggressively.
(2) Avoid medications that may trigger an attack.
(3) Start a dextrose infusion to provide carbohydrate load for prolonged fasting or dehydration.
(4) Treat an acute attack as usual except avoid a hematin infusion unless necessary.
A woman with porphyria who wants to avoid pregnancy should use a method other than oral contraceptives since these can trigger an attack.
